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帕金森病患者血液中的聚集抗性α-突触核蛋白四聚体减少。

Aggregation-resistant alpha-synuclein tetramers are reduced in the blood of Parkinson's patients.

机构信息

UK Dementia Research Institute, University College London, London, W1T 7NF, UK.

Institute of Aerospace Medicine, German Aerospace Center (DLR), 51147, Cologne, Germany.

出版信息

EMBO Mol Med. 2024 Jul;16(7):1657-1674. doi: 10.1038/s44321-024-00083-5. Epub 2024 Jun 5.

Abstract

Synucleinopathies such as Parkinson's disease (PD) are defined by the accumulation and aggregation of the α-synuclein protein in neurons, glia and other tissues. We have previously shown that destabilization of α-synuclein tetramers is associated with familial PD due to SNCA mutations and demonstrated brain-region specific alterations of α-synuclein multimers in sporadic PD patients following the classical Braak spreading theory. In this study, we assessed relative levels of disordered and higher-ordered multimeric forms of cytosolic α-synuclein in blood from familial PD with G51D mutations and sporadic PD patients. We used an adapted in vitro-cross-linking protocol for human EDTA-whole blood. The relative levels of higher-ordered α-synuclein tetramers were diminished in blood from familial PD and sporadic PD patients compared to controls. Interestingly, the relative amount of α-synuclein tetramers was already decreased in asymptomatic G51D carriers, supporting the hypothesis that α-synuclein multimer destabilization precedes the development of clinical PD. Our data, therefore suggest that measuring α-synuclein tetramers in blood may have potential as a facile biomarker assay for early detection and quantitative tracking of PD progression.

摘要

突触核蛋白病,如帕金森病(PD),其特征是α-突触核蛋白在神经元、神经胶质和其他组织中的积累和聚集。我们之前已经表明,由于 SNCA 突变导致的α-突触核蛋白四聚体的不稳定性与家族性 PD 有关,并根据经典的 Braak 传播理论,在散发性 PD 患者中证明了脑区特异性的α-突触核蛋白多聚体改变。在这项研究中,我们评估了具有 G51D 突变的家族性 PD 和散发性 PD 患者血液中细胞溶质α-突触核蛋白的无序和高级多聚体形式的相对水平。我们使用了一种适用于人类 EDTA-全血的体外交联方案。与对照组相比,家族性 PD 和散发性 PD 患者血液中的高级α-突触核蛋白四聚体的相对水平降低。有趣的是,无症状 G51D 携带者的α-突触核蛋白四聚体的相对数量已经减少,这支持了α-突触核蛋白多聚体不稳定先于临床 PD 发展的假说。因此,我们的数据表明,测量血液中的α-突触核蛋白四聚体可能具有作为早期检测和定量跟踪 PD 进展的简便生物标志物检测的潜力。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0f12/11250827/f00086521d78/44321_2024_83_Fig1_HTML.jpg

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